PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomes (Q35247414)

7 August 2017

title

PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomes (English)

1 reference

7 August 2017

Jeffrey Teckman

7

0 references

author name string

Christopher J Haddock

1

1 reference

7 August 2017

Keith Blomenkamp

2

1 reference

7 August 2017

Madhav Gautam

3

1 reference

7 August 2017

Jared James

4

1 reference

7 August 2017

Joanna Mielcarska

5

1 reference

7 August 2017

Edward Gogol

6

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7 August 2017

Dorota Skowyra

8

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7 August 2017

language of work or name

English

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publication date

11 September 2014

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7 August 2017

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7 August 2017

volume

9

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7 August 2017

issue

9

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7 August 2017

page(s)

e106371

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Creative Commons Attribution 4.0 International

7 August 2017

copyright license

start time

11 September 2014

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April 2022 Public Data File from Crossref

copyright status

copyrighted

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Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model

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Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency

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Crystal structure of a stable dimer reveals the molecular basis of serpin polymerization

12 July 2018

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The structural basis of serpin polymerization studied by hydrogen/deuterium exchange and mass spectrometry

12 July 2018

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Prions and the proteasome

12 July 2018

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Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model.

12 July 2018

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12 July 2018

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Extensive immunoglobulin production sensitizes myeloma cells for proteasome inhibition.

12 July 2018

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Mitochondrial dysfunction, free radical generation and cellular stress response in neurodegenerative disorders

12 July 2018

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The ubiquitin-proteasome system in Huntington's disease.

12 July 2018

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Characterization of an ERAD gene as VPS30/ATG6 reveals two alternative and functionally distinct protein quality control pathways: one for soluble Z variant of human alpha-1 proteinase inhibitor (A1PiZ) and another for aggregates of A1PiZ.

12 July 2018

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ATPase activity of p97/valosin-containing protein is regulated by oxidative modification of the evolutionally conserved cysteine 522 residue in Walker A motif

12 July 2018

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12 July 2018

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Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.

12 July 2018

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Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.

12 July 2018

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12 July 2018

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12 July 2018

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12 July 2018

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The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes

12 July 2018

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12 July 2018

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Impairment of the ubiquitin-proteasome system by protein aggregation

12 July 2018

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Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

12 July 2018

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Topography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational disease

12 July 2018

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12 July 2018

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Epoxomicin, a potent and selective proteasome inhibitor, exhibits in vivo antiinflammatory activity

12 July 2018

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12 July 2018

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12 July 2018

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A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency

12 July 2018

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Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice

12 July 2018

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12 July 2018

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12 July 2018

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12 July 2018

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The proteasome load versus capacity balance determines apoptotic sensitivity of multiple myeloma cells to proteasome inhibition.

26 September 2018

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Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4161314

Dose-dependent inhibition of proteasome activity by a mutant ubiquitin associated with neurodegenerative disease

26 September 2018

1 reference

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26 September 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4161314

Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4161314

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26 September 2018

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26 September 2018

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Impaired degradation of PKCalpha by proteasome in a cellular model of Huntington's disease

26 September 2018

1 reference

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Transglutaminase potentiates ligand-dependent proteasome dysfunction induced by polyglutamine-expanded androgen receptor

26 September 2018

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Parkin protects against the toxicity associated with mutant alpha-synuclein: proteasome dysfunction selectively affects catecholaminergic neurons

26 September 2018

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Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy

26 September 2018

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A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system

26 September 2018

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The serpins: nature's molecular mousetraps

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Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity

26 September 2018

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12 December 2020

Short-lived green fluorescent proteins for quantifying ubiquitin/proteasome-dependent proteolysis in living cells

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Retention of mutant alpha(1)-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response

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Autophagy: an ER protein quality control process

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THE SERUM ALPHA-L-ANTITRYPSIN IN FAMILIES WITH HYPO-ALPHA-L-ANTITRYPSINEMIA

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Importance of the different proteolytic sites of the proteasome and the efficacy of inhibitors varies with the protein substrate

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Liver Disease in Alpha1-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

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Differential impairment of 20S and 26S proteasome activities in human hematopoietic K562 cells during oxidative stress

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Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity

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The natural history of liver disease in alpha 1-antitrypsin deficient children

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Neonatal hepatitis induced by alpha 1-antitrypsin: a transgenic mouse model

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Changes of endoplasmic reticulum chaperone complexes, redox state, and impaired protein disulfide reductase activity in misfolding alpha1-antitrypsin transgenic mice

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12 December 2020

10.1371/JOURNAL.PONE.0106371

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DOI

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7 August 2017

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1 reference

7 August 2017

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